Naveen Kakkar

Red cell distribution width (RDW) in the diagnosis of iron deficiency with microcytic hypochromic anemia.

ObjectiveTo study the utility of red cell distribution width (RDW) in the diagnosis of iron deficiency among children with microcytic hypochromic anemia.Methods151 children (6 months-12 years) with microcytic (MCV<75 fl) anemia were classified into iron deficient (IDA) and non-iron deficient anemia (non-IDA) on the basis of serum ferritin and total iron binding capacity (TIBC). RDW values were obtained on an automated hematology analyzer. Receiver operator curves (ROC) were constructed and the utility of RDW in diagnosis of iron deficiency was studied.ResultsThe mean RDW value was 18.37±2.22% in IDA group (97 children) compared to 16.55±1.51 % in the non-IDA group (54 children) (p<0.0001, unpaired t test). In IDA group, the mean RDW value was 16.60±1.78%, 17.95±1.91% and 20.55±1.32% among mild, moderate and severely anemic children (p<0.0001, ANOVA test). The corresponding values in non-IDA group were 16.03±1.25%, 16.76±1.20% and 16.77±2.68% respectively (p=0.269, ANOVA test). At a cut-off value of 17.4%, as obtained from the ROC curve, the sensitivity and specificity of RDW in diagnosis of IDA were 81.0% and 53.4% and a positive and negative predictive value of 63.0% and 72.2% respectively.ConclusionRDW has a limited specificity for diagnosis of IDA among children with microcytic hypochromic anemia.

Erythropoietin use and abuse

Recombinant human erythropoietin (rhEPO) is arguably the most successful therapeutic application of recombinant DNA technology till date. It was isolated in 1977 and the gene decoded in 1985. Since then, it has found varied applications, especially in stimulating erythropoiesis in anemia due to chronic conditions like renal failure, myelodysplasia, infections like HIV, in prematurity, and in reducing peri-operative blood transfusions. The discovery of erythropoietin receptor (EPO-R) and its presence in non-erythroid cells has led to several areas of research. Various types of rhEPO are commercially available today with different dosage schedules and modes of delivery. Their efficacy in stimulating erythropoiesis is dose dependent and differs according to the patients disease and nutritional status. EPO should be used carefully according to guidelines as unsolicited use can result in serious adverse effects. Because of its capacity to improve oxygenation, it has been abused by athletes participating in endurance sports and detecting this has proved to be a challenge.

Red Cell Cytograms Generated by an ADVIA 120 Automated Hematology Analyzer: Characteristic Patterns in Common Hematological Conditions

Background: Graphical data output from automated hematology analyzers, especially those related to red blood cells, have been traditionally ignored in favor of the more frequently used numerical values. This study presents characteristic red cell cytograms generated by Advia-120, a 5-part automated hematology analyzer, as seen in common hematological conditions. Materials and Methods: Both numerical and graphical data output from Advia-120 automated hematology analyzer (Technicon H1 series) were studied over a period of 2 years from blood samples processed for routine complete blood count in a high output hematology laboratory of a tertiary care hospital. Data were correlated with relevant clinical information and red cell parameters to diagnose the hematological condition in patients with anemia and abnormal red cell indices/red cell cytograms. Results: Common hematological conditions like iron deficiency anemia, thalassemia, macrocytic anemia, dual deficiency anemia, cold agglutinins, and others were diagnosed based on characteristic red cell cytogram patterns, red cell indices, and relevant biochemical/other investigations. Conclusions: Red cell cytogram patterns from automated analyzers provide clinically useful information that acts as an adjunct to the numerical parameters and at times is even diagnostic of some hematological conditions not otherwise suspected.

Thrombotic thrombocytopenic purpura and systemic lupus erythematosus: successful management of a rare presentation.

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the critical care team. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. A patient critically ill with TTP and SLE, successfully managed in the acute period of illness with plasma exchange, steroids and mycophenolate mofetil is described.

Hemophilia treatment center: A stratification model for developing countries: A pilot study from India

Background: Hemophilia center, treatment center (TC), hemophilia TC (HTC), and Hemophilia Comprehensive Care Center (CCC) are terminologies used to describe centers caring for persons with hemophilia (PWH). These are based on their capability to provide multidisciplinary care and laboratory services. Widely described are the European HTCs (EHTCs) and the European Hemophilia CCCs (EHCCCs). However, most centers in developing countries providing care for PWH have variable clinical expertise and laboratory facilities, which do not qualify for the existing models. Materials and Methods: This cross-sectional study was done to evaluate the laboratory and clinical care facilities available in HTCs in India. The survey questionnaire was sent to 62 HTCs in India. Laboratory and clinical care facilities were categorized based on a predefined stratification model. Level IV being the minimum and Level I the maximum were used to define clinical and laboratory facilities. Results: Fifty-two (85%) centers responded representing 17 states in India. Only 28 HTCs had attached laboratory services. Although all the centers cared for acute bleeds, only half managed chronic joint disease (Level III) while one-sixth could perform surgeries (Level II). Only one-third of the laboratories had instituted quality control measures and performed factor assays. Only four centers qualified for EHTC criteria and two for the EHCCC criteria. Conclusion: This HTC stratification model provides assessment and differentiation of the clinical and laboratory services. It allows an individual HTC to identify the standard of care and provides a framework for objectively planning, implementing, and evaluating its services.

Spurious platelet count due to cryoglobulins in a patient with smoldering myeloma.

Use of automated hematology analyzers for routine blood count reporting has increased the reproducibility and accuracy of test results. However, at times, these instruments may generate spurious test results. Such results can result in inappropriate investigations or treatment decisions in patients. Spuriously normal or high platelet counts carry the risk of under diagnosis of the true thrombocytopenia with adverse clinical implications. We present a patient with smoldering myeloma with spurious platelet count due to cryoglobulins.

Neutrophil Aggregates in a Young Female With Sepsis

Patient 27-year-old woman. Chief Complaint High-grade fever and generalized rash for 2 days. Physical Exam Findings The patient was febrile and tachypneic with a generalized macular rash. Her pulse was 132/minute and she had a blood pressure of 90/70 mmHg. There was no organomegaly. She was clinically diagnosed to have sepsis due to toxic shock syndrome. Drug History On sodium valproate 500 mg twice a day. Medical History She has epilepsy and is currently on treatment. She had delivered a baby by Caesarian section 4 days earlier. Family History Unremarkable. Principal Laboratory Findings Table 1, Image 1 , and Image 2 . 1. What are this patient’s most striking clinical and laboratory findings? 2. How do you explain these findings? 3. What other clinical conditions have been associated …

Fever and Petechiae in a Middle Aged Male

1. What are this patient’s most striking clinical and laboratory findings? 2. How do you explain these findings? 3. What is this patient’s most likely diagnosis? 4. What is the causative organism of this patient’s disease? 5. What is the geographical distribution of the organism causing this patient’s disease? 6. What is the life cycle of the organism causing this patient’s disease? 7. What are the clinical features of the disease caused by this organism? 8. How is the laboratory diagnosis made of the disease caused by this organism? 9. What are other conditions in which atypical lymphocytes can be seen on a peripheral blood smear? Possible Answers

Jaundice and Cola-Colored Urine in a Young Indian Boy

1. What is (are) this patient’s most striking clinical and laboratory findings? 2. How do you explain these findings? 3. What is this patient’s most likely diagnosis? 4. What is the geographical distribution of this condition? 5. What is the inheritance pattern and the subtypes of this condition? 6. What is the pathogenesis of the condition leading to hemolysis in this patient? 7. What are the principal clinical features of this patient’s condition? 8. What are the factors which predispose an individual to the clinical manifestations associated with this patient’s condition? 9. What are the laboratory investigations which can be carried out to diagnose this patient’s condition? 10. What are the other red cell enzymopathies which have hematological manifestations?

Unrelated and related donor transplantation for beta-thalassemia major: A single-center experience from India

Hematopoietic stem cell transplantation (HSCT) remains the only curative treatment in patients with β‐thalassemia major. A matched sibling or a related donor is usually found in only 25%‐30% of the patients. There are limited data on matched unrelated donor (MUD) transplants from India. We reviewed HSCT outcome in 56 children with TM who underwent 57 transplants at our center. Related donor (RD) (n=43) and MUD (n=14) transplants were performed with TreoFluT‐based conditioning regimen in majority (95%) of patients. Peripheral blood stem cells (PBSC) were the preferred (85%) source of stem cells. The overall survival (OS) at 1 year in RD and MUD groups was 87.6±5.2% and 85.7±9.4% at a median follow‐up of 25 (1‐92) months and 22.5 (1‐50) months, respectively (P=.757). The thalassemia‐free survival (TFS) at 1 year was 87.6±5.2% and 77.1±11.7% with a median follow‐up of 24 (1‐92) and 16.5 (1‐50) months, respectively (P=.487). Although acute (14% vs 64%) and chronic graft‐versus‐host disease (GVHD) (13.9% vs 42.9%), infectious (39.5% vs 71.4%), and non‐infectious (37.2% vs 78.5%) complications are higher in MUD transplant group, the present data show a comparable OS and TFS among RD and MUD group with treosulfan‐based regimen using PBSC grafts.